ObjectiveTo explore the clinical characteristics and improve the knowledge of diagnosis and treatment of complex pulmonary arteriovenous fistula (PAVF) as well as enrich the experience of diagnosis and treatment of the disease.MethodsA retrospective analysis of pathogenetic process clinical manifestations, imaging features and diagnosis and treatment was conducted on one case of complex PAVF. The literature review was carried out with " complex pulmonary arteryovenous fistula (malformation)” as the research terms in English and Chinese respectively in CNKI, WanFang and PubMed database. Search time ranged from January 1997 to April 2018, and the literature was screened and reviewed.ResultsThe patient was a 47-year-old female complained of recurrent epistaxis for 40 years, intermittent hemoptysis for 20 years, headache, dizziness, chest pain, chest tightness for 4 years and the symptoms were aggravated by 3 months, visiting this hospital on January 23, 2018. Pulmonary CT angiography revealed multiple nodules in internal and external segment and outer basal segment of right lung, anterior basal segment and outer basal segment of left lung. CT enhanced scan showed that the thickened pulmonary artery was connected with the above lesion, and the edge was accompanied by large draining veins. Pulmonary artery revascularization showed complex PAVF abnormal branches. The diagnosis was complex PAVF, and interventional embolization therapy was carried out and curative effect was satisfactory during the follow-up. A total of 6 literatures were reviewed in above-mentioned databases, including 4 Chinese literatures and 2 English literatures, containing 10 patients, including 8 males and 2 females, with an average age of (9.7±7.0) years. Most of the clinical manifestations were shortness of breath after exercise, cyanosis and hemoptysis and all patients were cured and discharged after interventional embolization treatment except for 1 patient refused treatment.ConclusionsComplex PAVF is a very rare pulmonary vascular malformation. The clinical manifestations mainly include hypoxemia, dyspnea, hemoptysis, and the preferred treatment is interventional embolization, which has a satisfactory clinical effect at a short-term follow-up.